Addison's Disease

Addison’s Disease – Treatment Options

Introduction
Addison’s disease, or primary adrenal insufficiency, is a chronic endocrine disorder caused by destruction or dysfunction of the adrenal cortex, resulting in deficient production of glucocorticoids (cortisol), mineralocorticoids (aldosterone), and often adrenal androgens. The most common cause in developed countries is autoimmune adrenalitis, while tuberculosis and other infections remain common causes worldwide. Clinical features include fatigue, weight loss, hypotension, hyperpigmentation, salt craving, and risk of adrenal crisis under stress. Treatment is lifelong hormone replacement and management of acute decompensations.

1. Hormone Replacement Therapy (Lifelong Standard Treatment)

• Glucocorticoid replacement:

  • Hydrocortisone (15–25 mg/day orally in 2–3 divided doses).

  • Alternatives: Prednisolone or dexamethasone in selected patients.

  • Dose titrated to avoid both under- and over-replacement (monitor weight, BP, symptoms).

• Mineralocorticoid replacement:

  • Fludrocortisone (0.05–0.2 mg/day) to maintain sodium balance, blood pressure, and prevent hyperkalemia.

  • Salt supplementation may be required, especially in hot climates or heavy sweating.

• Adrenal androgen replacement (optional):

  • Dehydroepiandrosterone (DHEA) supplementation may improve mood, energy, and libido in some women.

2. Management of Adrenal Crisis (Life-Threatening Emergency)

• Presentation: Severe hypotension, shock, abdominal pain, vomiting, confusion, hypoglycemia, electrolyte disturbances.

• Immediate treatment:

  • IV hydrocortisone (100 mg bolus, then 50–100 mg every 6–8 hours).

  • Rapid IV fluids (0.9% saline, ± dextrose for hypoglycemia).

  • Correct electrolyte imbalances (especially hyperkalemia, hyponatremia).

  • Treat underlying trigger (infection, trauma, surgery).

3. Stress Dose Adjustment

• Mild illness or fever: Double or triple the usual glucocorticoid dose temporarily.

• Surgery or major stress: IV hydrocortisone (50–100 mg every 6–8 hours), taper postoperatively.

• Patient education: Carry medical alert identification and emergency steroid injection kit.

4. Monitoring and Long-Term Care

• Clinical monitoring: Fatigue, blood pressure, weight, skin pigmentation, and quality of life.

• Biochemical monitoring: Electrolytes, plasma renin activity (to titrate fludrocortisone).

• Avoid over-replacement: Prevents iatrogenic Cushing’s syndrome, osteoporosis, and metabolic complications.

• Bone health: Adequate calcium, vitamin D, and bone density monitoring.

5. Lifestyle and Preventive Measures

• Adherence to lifelong hormone replacement.

• Education on sick-day rules and emergency care.

• Adequate hydration and salt intake.

• Vaccinations and infection prevention to reduce risk of adrenal crisis triggers.

6. Multidisciplinary Care

• Endocrinologists: Diagnosis, hormone replacement adjustment, and long-term management.

• Primary care providers: Monitoring comorbidities and general health.

• Emergency physicians: Immediate recognition and treatment of adrenal crisis.

• Nurses, pharmacists, and patient educators: Training patients on stress dosing and emergency injection use.